Technical Data
B0290-07A
BCKDK, CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase, Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase)
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Technical Data |
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B0290-07A |
BCKDK, CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase, Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase) |
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Description: The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitiously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:1,000 Western Blot: 1:100-1:500 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. |
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